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Causes of pulmonary hypertension

Pulmonary Hypertension [Source]
Pulmonary hypertension is the narrowing of the pulmonary arterioles within the lung. The narrowing of the arteries creates resistance and an increased work load for the heart. The heart becomes enlarged from pumping blood against the resistance.

Some symptoms include chest pain, weakness, shortness of breath, and fatigue.

The goal of treatment is control of the symptoms, although the disease usually develops into congestive heart failure.


Pulmonary hypertension can be subdivided into
  • primary (idiopathic) and 
  • secondary types.
CXR: Pulmonary Hypertension. [Source]


Primary pulmonary hypertension (PPH)

Primary pulmonary hypertension (PPH) is a diagnosis of exclusion where no cause of pulmonary hypertension is discernible.

A number of theories have been put forward to explain the origin of PPH; none has yet gained ascendancy.

Primary Pulmonary Hypertension (PPH): A Diagnosis of Exclusion. [Source]

Secondary pulmonary hypertension

Secondary pulmonary hypertension can be caused by:
  • vasoconstriction due to chronic hypoxia;
  • loss of pulmonary vessels due to chronic obstructive pulmonary disease, pulmonary fibrosis, or vasculitis; 
  • pulmonary thromboembolic disease; 
  • congenital left-to-right shunting;
  • left heart failure; and 
  • mitral valve disease. 
Major Causes of Secondary Pulmonary Hypertension by Mechanisms. [Source]

Further reading:
  1. McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al: American College of Cardiology Foundation Task Force on Expert Consensus Documents; American Heart Association; American College of Chest Physicians; American Thoracic Society, Inc; Pulmonary Hypertension Association. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians: American Thoracic Society, Inc; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53:1573-1619.
  2. Rich S. Pulmonary hypertension. In: Bonow ROL, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 78.

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